Apolipoproteins and Lipoprotein Families in Familial Lecithin: Cholesterol Acyltransferase Deficiency

P. Alaupovic Lipoprotein Laboratory, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Institute of Medical Genetics, University of Oslo, and Medical Dept. A, University of Oslo, Rikshospitalet, Oslo, Norway

W. J. McConathy Lipoprotein Laboratory, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Institute of Medical Genetics, University of Oslo, and Medical Dept. A, University of Oslo, Rikshospitalet, Oslo, Norway

M. D. Curry Lipoprotein Laboratory, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Institute of Medical Genetics, University of Oslo, and Medical Dept. A, University of Oslo, Rikshospitalet, Oslo, Norway

H. N. Magnani Lipoprotein Laboratory, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Institute of Medical Genetics, University of Oslo, and Medical Dept. A, University of Oslo, Rikshospitalet, Oslo, Norway

H. Torsvik Lipoprotein Laboratory, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Institute of Medical Genetics, University of Oslo, and Medical Dept. A, University of Oslo, Rikshospitalet, Oslo, Norway

K. Berg Lipoprotein Laboratory, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Institute of Medical Genetics, University of Oslo, and Medical Dept. A, University of Oslo, Rikshospitalet, Oslo, Norway

E. Gjone Lipoprotein Laboratory, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Institute of Medical Genetics, University of Oslo, and Medical Dept. A, University of Oslo, Rikshospitalet, Oslo, Norway

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Abstract

The plasma lipoprotein system in patients with familial LCAT deficiency contains all the polypeptides and lipoprotein families identified previously in plasma lipoproteins of normal subjects. There are, however, some qualitative and quantitative differences. The most significant qualitative difference is the presence in LCAT-deficient plasma of LP-X, the abnormal low density lipoprotein species characteristic of obstructive jaundice. The major quantitative difference is the reduced concentration of ApoA, ApoB, and ApoD in LCAT-deficient plasma. It has already been demonstrated that most lipoprotein species in LCAT deficiency have an abnormal lipid composition characterized by unusually high contents of unesterified cholesterol and lecithin and a negligible amount of esterified cholesterol (4). These abnormalities of the lipoprotein system in familial LCAT deficiency seem to result from the virtual absence of LCAT activity rather than from the absence of normally occurring lipoprotein families or their constitutive polypeptides.

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Apolipoproteins and Lipoprotein Families in Familial Lecithin: Cholesterol Acyltransferase Deficiency

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Apolipoproteins and Lipoprotein Families in Familial Lecithin: Cholesterol Acyltransferase Deficiency

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